Uçsel R, Savaşan S, Coban A, Metin F, Can G
Department of Pediatrics, Istanbul University Faculty of Medicine.
Turk J Pediatr. 1996 Apr-Jun;38(2):257-60.
Factor VII deficiency is a rare congenital coagulopathy. Prolonged prothrombin time with normal partial thromboplastin time indicates factor VII deficiency. For the definitive diagnosis, the specific factor VII level should be investigated. We report a seven-day-old, male, full-term newborn who was admitted with the diagnosis of sepsis. Hematological tests revealed prolonged prothrombin time and a factor VII level of five percent. After antibiotic therapy and fresh frozen plasma replacement his clinical status improved but the prothrombin time continued to be prolonged. On the 14th day, just before the end of antibiotic therapy, the infant died of sudden intracerebral hemorrhage. In this article, the clinical features and management of factor VII deficiency are discussed.
凝血因子 VII 缺乏症是一种罕见的先天性凝血病。凝血酶原时间延长而部分凝血活酶时间正常提示凝血因子 VII 缺乏。为明确诊断,应检测特定的凝血因子 VII 水平。我们报告一例 7 日龄、足月、男性新生儿,因败血症入院。血液学检查显示凝血酶原时间延长,凝血因子 VII 水平为 5%。经抗生素治疗和输注新鲜冰冻血浆后,其临床状况有所改善,但凝血酶原时间仍持续延长。在第 14 天,就在抗生素治疗即将结束前,该婴儿死于突发性脑出血。本文讨论了凝血因子 VII 缺乏症的临床特征及治疗。